Atypical anti-glomerular basement membrane disease with anti-GBM antibody negativity and ANCA positivity: a case report

Na Guo,Song Lei,Qinghua Yin,Yanjun He,Ping Fu

doi:10.1186/s12882-021-02232-1

Na Guo, Song Lei + Show 3 more

Open Access

https://doi.org/10.1186/s12882-021-02232-1

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Abstract

BackgroundAnti-glomerular basement membrane (anti-GBM) disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure. Classic cases of anti-GBM disease are diagnosed based on the presence of the anti-GBM antibody in serum samples and kidney or lung biopsy tissue samples. However, atypical cases of anti-GBM disease are also seen in clinical practice.Case presentationWe herein report the rare case of a patient with atypical anti-GBM disease whose serum was negative for the anti-GBM antibody but positive for the myeloperoxidase (MPO) anti-neutrophil cytoplasmic antibody (p-ANCA) and another atypical ANCA. Laboratory test results showed severe renal insufficiency with a creatinine level of 385 μmol/L. Renal biopsy specimen analysis revealed 100% glomeruli with crescents; immunofluorescence showed immunoglobulin G (IgG) linearly deposited alongside the GBM. Finally, the patient was discharged successfully after treatment with plasmapheresis, methylprednisolone and prednisone.ConclusionThis patient, whose serum was negative for the anti-GBM antibody but positive for p-ANCA and another atypical ANCA, had a rare case of anti-GBM disease. Insights from this unusual case might help physicians diagnose rare forms of glomerulonephritis and treat affected patients in a timely manner.

Highlights

Anti-glomerular basement membrane disease is an organ-specific autoimmune disease that involves the lung and kidneys and leads to rapid glomerulonephritis progression, with or without diffuse alveolar hemorrhage, and even respiratory failure
This patient, whose serum was negative for the anti-GBM antibody but positive for p-Anti-Neutrophil Cytoplasmic Antibody (ANCA) and another atypical ANCA, had a rare case of anti-GBM disease
Edema and skin rashes in both lower limbs appeared 3 days after this treatment. She was further treated for edema with furosemide and spironolactone at the same local hospital, and she was transferred to our hospital in September 2019

Summary

Conclusion

This patient, whose serum was negative for the anti-GBM antibody but positive for p-ANCA and another atypical ANCA, had a rare case of anti-GBM disease.

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Discussion and conclusion